The role of exocrine pancreatic insufficiency in HIV-associated steatorrhea and malnutrition is undefined. Not infrequently steatorrhea in people with HIV infection remains unexplained. A clinically significant proportion of children with HIV infection and steatorrhea may have exocrine pancreatic insufficiency (PI). Therefore this study was conducted to examine the prevalence of steatorrhea and PI and their association with growth, nutritional and immune status parameters in a sample of children with perinatally acquired HIV infection. Subjects had weight (kg), height (cm) and skinfold thicknesses measured. HIV-RNA viral load (copies/ml), CD-4 counts biochemical evidence of hepatobilary disease (elevated bilirubin and/or hepatic transaminases) within 3-months of stool sample collection were obtained from the medical record. Stool samples were analyzed for steatorrhea using the Sudan III qualitative test. Screening for PI was conducted using the fecal elastase-1 enzyme (FE-1) ELISA. Subjects with FE-1 levels in the range for PI (< 200 mg/g) had confirmatory testing with 72-hour stool and diet collection and the hormone-stimulated pancreatic function test. Z-scores were computed for height (HAZ), weight (WAZ), triceps skinfold (TSFZ) and upper arm muscle area (UAMAZ). Data was analyzed using two sample t-tests and Pearson Chi square. Statistical significance was defined as p 0.05).